CTD-ILD; Sjorgren’s Syndrome, polymyositis; Antisynthetase syndromePosted by marisa-beard on July 21, 2021 at 8:33 am
Greetings — I am new to the forum and new to all things wrong with me. Just curious if anyone else out there in forum land has the same or similar diagnosis as me. I am on oxygen 24/7, and I use a POC and at-home concentrator. Although I hate hauling the oxygen around with me, I would not be alive without it. I am currently on 20 mg of prednisone and 30oo mg of CellCept (mycophenolate) – I just increased the CellCept a day ago, so I am trying to get used to the increase. I have gained 25 lbs since I started taking prednisone (the CellCept could also add to that weight gain). I am careful of what I eat, but the weight keeps going up — very frustrated. My last PFT wasn’t very good — I had one in Feb and another one in June. All of my numbers had gone down.
FEV1 – JUNE 57% ( FEB 67%)
FVC – JUNE 52% (FEB 60%)
Absolute FEV1 /FVC ratio – JUNE 88% (FEB 88%)
TLC – JUNE 43%
RV – JUNE 30%
DLCO – JUNE 39%
As one of my pulmonologists put it, the autoimmune disorders are attacking your lungs. We need to try and get that under control before considering a lung transplant.
I learn something new every week (sometimes every day). I need to wear a Holter Monitor for 7 days, which I did wear for 4 days until it quit working. I get to start over this week. As a point for more information, I am 62 years old, and I still work full-time. I am blessed to work for a university that allows me to work from home when I need to (which is 10-30 hours a week, depending on my energy level.)
I would love to hear from anyone who has the same or similar diagnosis as me.
MemberJuly 21, 2021 at 1:43 pm
I wanted to welcome you to the forum. Unfortunately, I don’t have answers to any of your questions as I have IPF and on oxygen on exertion. I understand about your numbers going down. One of mine PFT numbers took a drastic drop in February so oxygen was added. Hopefully, someone on the forum will have some answers to your questions. Hang in there.
MemberJuly 21, 2021 at 7:05 pm
Just to clarify what my diagnoses (listed in the subject area) are Connective Tissue Disease- Interstitial Lung Disease(CTD-ILD) basically brought on by these 3 autoimmune disorders Sjogren’s Syndrome, polymyositis, and Antisynthetase syndrome. I am currently under the care of a rheumatologist, pulmonologist, lung transplant pulmonologist, ophthalmologist, neurologist, and possibly a cardiologist (still undergoing a Holter study). I am on oxygen 24/7 using a large concentrator while at home and a portable concentrator when I am out and about. Usually 2L while at rest at home but up to 4L if is am doing something. When I am out and moving I can range from 3-6L I am taking 2o mg of prednisone, 3000 mg of mycophenolate (CellCept), <span style=”font-weight: 400;”>Sucralfate (heartburn/GERD), Xiidra eyedrops (dry eyes from Sjogren’s), Refresh gel drops (dry eyes from Sjogren’s), Potassium, Magnesium, and a few other things. I am not on the lung transplant list at this time — trying to get the attack on the lungs a little more under control. I hope this is helpful. </span>
MemberJuly 22, 2021 at 9:08 am
Welcome, I have usual interstitial pneumonia. Had a lung biopsy that turned into a thoracotomy and discovered I had connective disease around the lungs. 5 years after my diagnosis I am still here. DLCO basically the same 10.4 March 2018 and 10.5 March 2021. Fev1 March 1.49 and Fev1 march 2021 1.27. I had a blood test for inflammation in 2018 and was high and after doing laser my blood test for inflammation is normal. I do laser on my lungs twice a week, and prednisone 10 mg. Lately I have lost weight, stopped drinking and eating after dinner. I take Nexium 20 mg twice a day ( article published on this web site of benefits of Esopremazole from Baylor university ). I also take a multivitamin. Use 2 liters of oxygen at night and portable unit when walking distances. Try to walk 2 miles a day 3 or 4 times a week. Good luck.
MemberJuly 22, 2021 at 2:48 pm
John, a couple of questions: what is a laser treatment for your lungs? And is that to lower inflammation? How does it do that? Thanks.
MemberJuly 22, 2021 at 3:27 pm
I would be interested in knowing about the laser treatment also!
MemberJuly 23, 2021 at 7:38 am
The laser is a un approved treatment that many people use. More information can be found on this site under laser. Andy Hall is a chiropractor with pulmonary fibrosis who is very knowledgeable about laser. He can be found on this web site or on Facebook laser group. The gerd is something to really watch out for. Gerd can be silent and you do not know you are suffering from gerd. Many people do supplements, I do laser. I do not think the laser stops the disease but like the prescription medications I think it slows the disease by reducing or eliminating the inflammation.
MemberJuly 22, 2021 at 3:12 pm
Hi Marissa, sorry to hear what all is going on with you. I have gone through pretty much the same testing since when diagnosed with CTD-ILD from polymyositis back in 2015. Although, my pulmonary fibrosis started several years before the polymyositis hit me. At any rate, I don’t know all my levels, but I’m on oxygen 24/7 at 4 lpm rest and 6 lpm when active, and just went on oxygen in February after a bout of double pneumonia. I use a concentrator (the airplane, I call it, because it’s noisy), a POC to drive since I’m just sitting, and etanks to go out and about, which is a major, major pain but necessary if I want to get out. I take 2000 mg of cellcept w/o problems. And my docs tell me I am in great condition except for my lungs. I have been on a lung transplant list since last November. Unfortunately, I have a high number of antibodies which makes finding a donor harder, but I am in the process of trying to multi-list at another transplant hospital to expand my field since my time for a transplant is narrowing (here the one I’m with stops transplanting at 75). I am 73, and I still work too; I teach community college courses online, which, like you, has worked out great since I have gone on oxygen. I have to say that I have found this whole experience so far to be a hard, hard road to follow, but since I have no choice, I try to stay active, go out with friends (despite the tanks), and wait for the call for the transplant. It’s the “new normal,” as I was told. So, that’s me w/many similarities to you. You are definitely not alone. Good luck to you in all your testing; I hope it all goes well. Anita
MemberJuly 22, 2021 at 3:35 pm
Your diagnosis does sound very similar to mine. I agree it is the “new normal” and at times hard to adjust to it. I do go to pulmonary rehab right now, which has been helpful to make me feel a little better. But when I walk a speedy 1.5 mph and use 3L of oxygen, it can also get depressing! I go into the office for at least 15 hours a week (I always aim for more, but it doesn’t usually happen) — I need to be around people! Since I am new to all of this (officially diagnosed in May of this year), what do you mean when you say antibodies? I assume this is something that is found out from all the blood work that is done. I am slowly learning what everything means, but medical terms are not my fortet. Marisa
MemberJuly 24, 2021 at 2:22 am
Hi Marisa – I do share some diagnosis with you. I was diagnosed with the ILD – IPF in late 2014. In early 2015 a second opinion and further tests indicated that I actually had Polymyositis first that resulted in Pulmonary Fibrosis. It was a shock to receive such a life changing diagnosis.
I live in Australia so at my age a lung transfer is not an option. Primary medications are / were Tacrolimus, Azathioprene and IViG. I am not on oxygen.
Your fortunate you can maintain employment. At times I could not walk or talk so had to retire. The medications worked well for me up to this year when I had an episode of increased active Pulmonary Fibrosis. This was treated with very high dose Prednisone ( in Hospital) and on discharge approval for Rituximab treatment.
Its incredible how the autoimmune diseases target specific organs or parts of our body. It’s also great that research is making inroads into our conditions.
My doctors advised I could have died in 2015 and I’m so grateful to still be here to have met my grandchildren.
I wish you every opportunity for improved health. Don’t sweat the small stuff. I found in my darkest hours I always maintain ‘Hope’ – it helped me through. Fay
MemberJuly 24, 2021 at 2:30 pm
Hi Fay —
I am blessed to work at a university that has allowed me to work at home as much I need to. I try to go in 3 mornings a week but I can only do so because of my oxygen. I am 62, so mentally, I was not ready to retire and so glad I have not had to — yet. I also teach online doctoral courses, so of course, I can do that from home!
I am learning not to sweat the small stuff. I have had such awesome support from my family but also my church.
I have a husband, 3 adult children, and 9 grandchildren, and I hope to be around for a while longer to make some memories!
It will be intr3esting to see what the PFT and Xrays show in Sept. At some point, they will do another CT scan. I am not sure when that will happen, but it will certainly help show whether I am in decline or holding my own.
MemberJuly 31, 2021 at 11:26 am
I had an appointment with my rheumatologist yesterday. I guess the 3000 mg of CellCept is doing its thing since my blood work shows the inflammation is down. She had a conversation with the transplant doctor (one of my pulmonologists) and he firmly believes that I need a lung transplant but I have to lose 35 lbs to even be considered. I did start reducing my prednisone by 2.5 mg today. I’ll do that for 2 weeks and if I tolerate that education I’ll reduce it by 2.5 mg more. I am hopeful that by reducing the prednisone that my weight loss attempts will be successful. I am also trying to purposefully walk more outside — it is pretty tough due to the hot weather.
MemberJuly 31, 2021 at 12:06 pm
Marisa, good to hear the cellcept is doing its job, and reducing the prednisone should help you lose the weight you need to lose. Walking in the heat is hard; I have the same problem here where I live, but I am in the process of convincing myself to get up earlier and walk in the cool mornings. Though certainly awake early, getting up is another story! In the meantime, though, I have started pulmonary rehab which is meant to help build up body strength; check to see if you can get into such a program. I think they are offered at every hospital? Good luck.
MemberAugust 2, 2021 at 7:34 am
My experience with pulmonary rehab has been great. I have been going approximately 3 times a week for 6 weeks. I have 6 more weeks on the program but can continue to go once I am officially done (they just won’t’ monitor me one on one). My treadmill experience is I am at 1.5 mph for 14 minutes on 3L of oxygen. 1.5 is mighty slow but at least I am moving. The rest of the machines aren’t too difficult for me most days! It will be interesting to see what happens today since the reduction of prednisone. I feel like I have no energy today but I did go into my office this morning so we’ll see how I survive!
MemberAugust 4, 2021 at 5:48 pm
I too share some of your diagnosis. I started noticing trouble breathing in 2019. At the time I lived at 6500′ elevation, and was a forest educator, enjoying hiking, skiing, and a very active lifestyle. After 10 months of testing, I was diagnosed with PF and ILD caused by Sjorgren’s syndrome. I don’t have any symptoms of the Sjorgren’s except for the PF. I started with 60 mg of prednisone in Oct 2019, and immediately gained 20 lbs. After 18 months, I am now completely tapered off of the prednisone, and have lost 10 of the extra lbs, but the next 10 lbs are stubborn! I, too, am on 3000 mg cellcept per day for the inflammation, and hydroxychloroquine for the Sjorgrens. I, too, completed the 7 week pulmonary rehab program. This program is saving my life! At first I could only do 1.8 mph, and was completely out of breath. I wanted to crumble in a ball and cry. I have continued the “maintenance” exercise program 3 times a week for 45 minutes each time. I am now up to 3.6 mph and 5% incline, with no oxygen at sea level (had to move away from my beloved mountains!). I have also started slow jogging at 4.2 mph, 0% incline, with O2 (6 minutes is the most I have jogged so far). It is a slow progress, and every time my meds change, I have to go backwards a little bit, then work back up to my former level for a week or two. I have learned not to push too hard and accept the small improvements. Keep up with your “maintenance” program – it will help you with the transplant and it will keep all your other supporting muscles in good shape to pull you through your next steps. Stay strong!
MemberAugust 5, 2021 at 3:24 pm
I appreciate your reply and sharing your experience. It has been so helpful to hear what others are going through, especially if there are similarities to my situation. I am ready to have my next pulmonologist appointment so I can at least have PFT done again. This is where there has been such a significant decline for me so it would be great to see it level off a little (give me more hope!). I see that you are taking hydroxychloroquine for Sjorgren’s syndrome. Does this help the eye dryness? I have been using the eyedrops Xiidra but my insurance won’t pay for them (as of yet). I tried Ristasin but the drops hurt my eyes so I discontinued. My prednisone taper is going pretty well (granted, I only lowered it by 2.5 mg) — I am thankful for that. I am considering purchasing a treadmill so I can increase my walking.
MemberAugust 6, 2021 at 12:46 am
The hydroxycholorquine has kept the Sjorgrens at bay. I haven’t had any other symptoms such as dry eye or dry mouth, just the extreme inflammation in the lungs. I don’t really understand how/why it attacked my lungs, and didn’t feel the damage happening until it was really severe. My rheumatologist says that “you will know” when the Sjorgrens comes back, so I guess that means I will feel like I have pneumonia again.
It’s good to taper off slowly from the prednisone. I was on it for such long time and when the pulmonologist tried to taper me off quickly, I had no energy and had a hard time breathing so we went back to 10 mg and tapered off 2.5 each month until I was down to 2.5 mg, and then went every other day for 2 weeks at 2.5 mg until I was at 0. I had no side effects from the slow taper, whew!
Yes a treadmill at home is a good idea, or whatever you can do to keep you going, even if it is minimal, at least you are moving! I have been walking outside on the days I don’t go to pulmonary maintenance. I am also finding that if I don’t exercise 5-7 times a week, I can’t lose that extra weight, and I quickly lose my fitness level if I take too many days off. My whole life is managing my disease these days!
Take care and I’m hoping for stability for you for your next PFT!
MemberAugust 6, 2021 at 7:24 am
Thanks for the info — I also have polymyositis and antisynthetase that have contributed to my lung condition (I have significant scarring). Thanks for the encouragement on exercising every day — that needs to be a new goal for me. I agree that the slow taper is the best thing to do for prednisone. That last time the doc (PCP) tried to get me to taper he did in 5 mg — way too fast for this body!
Have a great day!
ModeratorAugust 5, 2021 at 6:58 pm
@joybarney888gmail-com Joy, thank you for sharing your story.. what an amazing testament to the power of pulmonary rehab!! Going from a tough 1.8 mile walk to JOGGING for six minutes is incredible. Keep up the good work, and keep telling it to us. We love to hear it 🙂
@marisa For both of you, I have seen Sjogren’s come up several times in the forums here. You might search the keyword “Sjogren’s” in our search bar here and find more posts from others who have both. Also, did you know the same company that publishes this website has a similar one for Sjogren’s? You can find it here, if you’re interested 🙂 If I were a medical researcher I would be thinking about the links between autoimmune conditions and PF. I find it fascinating and I feel like there is not enough focus on it in research.
MemberAugust 5, 2021 at 9:03 pm
Thanks so much for the information!
MemberAugust 6, 2021 at 12:29 am
Christie, thank you so much for the information! I will definitely check out the other site and search Sjorgrens here. Thank you also for the encouraging words!
Log in to reply.